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Brief Report
October 2018

Radial Hemorrhage in Henle Layer in Macular Telangiectasia Type 2

Author Affiliations
  • 1Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California, Los Angeles
  • 2New England Eye Center, Tufts University School of Medicine, Boston, Massachusetts
  • 3Greater Los Angeles VA Healthcare Center, Los Angeles, California
JAMA Ophthalmol. 2018;136(10):1182-1185. doi:10.1001/jamaophthalmol.2018.2979
Key Points

Question  Is radial hemorrhage in the outer plexiform layer of Henle characteristic of deep retinal capillary plexus (DCP) pathology, as in macular telangiectasia type 2 (MacTel 2)?

Findings  In this case series, 3 patients with sudden vision loss presented with characteristic radial hemorrhage in Henle layer. Optical coherence tomography angiography illustrated microvascular abnormalities in the DCP consistent with MacTel 2 in the absence of subretinal neovascularization.

Meaning  MacTel 2 is associated with microvascular abnormalities in the DCP pathology and may be complicated by sudden radial hemorrhage in the outer plexiform layer of Henle independent of subretinal neovascularization.

Abstract

Importance  Radial hemorrhage in the outer plexiform layer of Henle may be a complication of macular telangiectasia type 2 (MacTel 2) and may occur because of microvascular abnormalities of the deep retinal capillary plexus in the absence of subretinal neovascularization.

Objective  To describe the multimodal imaging findings, including cross-sectional and en face optical coherence tomography (OCT), of radial hemorrhage in the outer plexiform layer of Henle, which may be a complication of MacTel 2.

Design, Setting, and Participants  This retrospective case series from 2 tertiary referral centers (Stein Eye Institute, Los Angeles, California; New England Eye Center, Boston, Massachusetts) between January 1, 2012, and December 31, 2017, describes 3 patients with MacTel 2 complicated by characteristic radial hemorrhage in the outer plexiform layer of Henle.

Main Outcomes and Measures  Color fundus photography, cross-sectional and en face OCT, OCT angiography (OCTA), fundus autofluorescence, and fluorescein angiography.

Results  Three male patients presented with sudden vision loss in the right eye. A characteristic radial pattern of hemorrhage was noted with color fundus photography. Cross-sectional and en face OCT and OCTA localized the hemorrhage to the outer plexiform layer of Henle in the absence of subretinal neovascularization. Optical coherence tomography findings consistent with MacTel 2 were identified in the fellow eye in each patient. At the follow-up visit 1 to 2 months after presentation, spontaneous resolution of the hemorrhage was noted in all 3 patients, and OCTA illustrated underlying microvascular abnormalities in the deep retinal capillary plexus in 2 patients.

Conclusions and Relevance  This report describes 3 patients with MacTel 2 complicated by characteristic radial hemorrhage in the outer plexiform layer of Henle, which may represent a characteristic finding in MacTel 2 that may develop as a result of microvascular abnormalities of the deep retinal capillary plexus in the absence of subretinal neovascularization.

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